We would like to thank Pellicelli (Gut 2004;53:1721) for his interest in our paper on portopulmonary hypertension in cirrhosis with refractory ascites (Gut 2003;52:1355–62).

We acknowledge that our proposal suggesting the role of endothelin 1 (ET-1) in the pathogenesis of portopulmonary hypertension is based on a small number of patients. However, the strong association between the elevated ET-1 levels in the pulmonary artery and the presence of portopulmonary hypertension in these patients would suggest that this is indeed the case. The confirmatory test would be a reduction in pulmonary pressure following blockade of endothelin receptors in the pulmonary circulation. However, this has not …